INTRODUCTION

Plasma cell neoplasms are characterized by the neoplastic proliferation of a single clone of plasma cells, typically produc-ing a monoclonal immunoglobulin. Plasma cell neoplasms can present as a single lesion (solitary plasmacytoma) or as multiple lesions (multiple myeloma). Solitary plasmacytomas most frequently occur in the bone (plasmacytoma of bone), but can also be found outside the bone in soft tissues (extra-medullary plasmacytoma).1 Approximately 5% of all cases of plasma cell disorders are solitary plasmacytomas of the bone.2 However, the involvement of the pancreas is rare. We report a case of an extramedullary plasmacytoma of the pancreas di-agnosed using ultrasonography-guided fine needle aspira-tion (EUS-FNA) with a review of the literature.

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CASE REPORT

A 58-year-old woman was transferred to our hospital for further evaluation of pelvic pain that was aggravated by walk-ing and began approximately 2 months before admission. The initial laboratory tests showed a hemoglobin level of 10.6 g/dL, and blood urea nitrogen was 12.0 mg/dL. The results of liver function tests were within normal limits. The level of to-tal protein level was 9.7 g/dL and the albumin level was 3.3 g/dL. A peripheral blood cell smear revealed mild lymphope-nia. Skeletal surveys detected a small osteolytic lesion with-out a sclerotic rim in the left parietal bone and a large bone destructive osteolytic lesion in the right inferior pelvic bone (Fig. 1). Incisional biopsy of the right pelvic bone suggested a malignant bone tumor. The lesion was diagnosed as a plas-macytoma. Further evaluation showed a serum free light chain lambda level of 3,475.3 mg/L and a β2-microglobulin level of 4.6 mg/L. Immunoelectrophoresis showed the pres-ence of an abnormal band of immunoglobulin G and lambda lanes. Plasma cells were 14.2% on bone marrow biopsy. Con-trast-enhanced abdominal computed tomography (CT) re-vealed a suspicious ill-defined marginated mass in the body of the pancreas (Fig. 2). To further characterize the lesion, magnetic resonance imaging (MRI) of the pancreas was per-formed. T2-weighted MRI indicated that the pancreatic prox-imal body contained a mass of subtle high signal intensity (Fig.

CASE REPORT

Extramedullary Plasmacytoma of the Pancreas Diagnosed Using Endoscopic Ultrasonography-Guided Fine Needle Aspiration

Young Hoon Roh1,2, Sang Youn Hwang1,2, Seon-Mi Lee1,2, Jung Woo Im1,2, Joon Suk Kim1,2, Kyeong A Kwon3, Joo Yeon Song4 and Soo Yeong Jeong4

1Gastrointestinal Cancer Center, Departments of 2Internal Medicine, 3Pathology, and 4Hemato-oncology, Dongnam Institute of Radiological & Medical Sciences, Busan, Korea

Extramedullary plasmacytoma involves organs outside the bone marrow; however, involvement of the pancreas is rare. We recently ex-perienced a case of extramedullary plasmacytoma of the pancreas that was diagnosed by endoscopic ultrasonography-guided fine needle aspiration (EUS-FNA). EUS-FNA, which has a high diagnostic accuracy and an excellent safety profile, is the modality of choice for es-tablishing tissue diagnosis. We report a case of extramedullary plasmacytoma of the pancreas diagnosed using EUS-FNA.

Key Words: Extramedullary plasmacytoma; Pancreas; Endosonography; Biopsy, fine-needle

Open Access

Received: April 16, 2013 Revised: June 26, 2013Accepted: July 20, 2013Correspondence: Sang Youn HwangGastrointestinal Cancer Center, Department of Internal Medicine, Dongnam Institute of Radiological & Medical Sciences, 40 Jwadong-gil, Jangan-eup, Gi-jang-gun, Busan 619-953, KoreaTel: +82-51-720-5114, Fax: +82-51-720-5992 E-mail: mongmani@hanmail.netcc This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Print ISSN 2234-2400 / On-line ISSN 2234-2443

http://dx.doi.org/10.5946/ce.2014.47.1.115

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Extramedullary Plasmacytoma of the Pancreas Diagnosed by EUS-FNA

3A) with marked diffusion restriction (Fig. 3B). For the histo-pathologic diagnosis, EUS-FNA was planned. Linear EUS (EU-ME1 Ultrasound System; Olympus, Tokyo, Japan) (GF-UCT 240; Olympus) revealed a 1.2×1.0-cm sized, hypoechoic, heterogeneous, well-defined round mass in the pancreatic body (Fig. 4A); FNA was performed via a transgastric ap-

Fig. 1. Pelvic magnetic resonance imaging shows a large bone destructive mass with a soft tissue mass in the right inferior pelvic bone (arrow).

Fig. 2. Contrast-enhanced abdomen-pelvis computed tomogra-phy shows a suspicious ill-defined marginated mass in the body of the pancreas (arrow).

Fig. 3. T2-weighted magnetic resonance imaging with a diffusion weighted image shows that the pancreatic proximal body contains (A) a mass of subtle high signal intensity with (B) marked diffusion restriction (arrows).

A B

Fig. 4. (A) Endoscopic ultrasound shows a hypoechoic and heterogenous mass in the body of the pancreas. (B) Fine needle aspiration was performed and samples were obtained for pathological analysis.

B A

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proach and five passages were made with a 22-gauge needle (EchoTip Ultra, ECHO-22; Cook Endoscopy, Winston-Salem, NC, USA) (Fig. 4B). The cytopatholgy results showed a small cell neoplasm and the immunohistochemical profile was com-patible with plasmacytoma (Fig. 5). The patient began com-bination therapy consisting of bortezomib, mephalan, and prednisolone with local radiation therapy for the right pelvic bone lesion. The patient was followed up carefully.

DISCUSSION

Multiple myeloma is a disease characterized by malignant proliferation of plasma cells typically involving medullary bones. Myelomatosis, a solitary bone myeloma, or an extra-medullary plasmacytoma are possible presentations. Extra-medullary plasmacytomas represent 3% to 4% of all plasma cell neoplasms and have a male predominance, with a three to five times higher incidence in men than in women; they usually occur in the sixth and seventh decades of life. Ap-proximately 80% to 90% of tumors develop in the head and neck area, although gastrointestinal involvement has been reported in 10% of cases. Pancreatic involvement of myeloma is relatively rare, with an incidence rate of 2.3% based on au-topsy studies. However, previous studies failed to describe the type of disease entity, and did not distinguish between multi-ple myeloma, myelomatosis, solitary bone myeloma, or extra-medullary plasmacytoma.3-6

The radiological differentiation of extramedullary plasma-cytoma of the pancreas from other pancreatic tumors such as poorly differentiated pancreatic neoplasm, lymphoma, and metastasis is difficult. Most cases of plasma cell infiltration of the pancreas are microscopic, and well-formed masses are un-usual. The latter may present as a focal mass or a diffuse en-largement of the pancreas; typically, the patient presents with jaundice and abdominal pain related to the obstruction of the biliary tree because pancreatic plasmacytoma is often lo-

cated in the head of the pancreas. In the present case, the mass was detected incidentally because it was located in the body of the pancreas. On ultrasound, pancreatic plasmacy-toma appears as a multilobulated heterogenous or a homog-enous hypoechoic mass. The CT features have been described as a focal multilobulated solid hypodense mass with homoge-neous intravenous contrast enhancement or diffuse enlarge-ment of the pancreas. MRI features include pancreatic en-largement with a lobulated contour, lower signal intensity than that of the liver on T1-weighted images, and diffusely in-creased signal intensity on T2-weighted pulse sequences with heterogeneous enhancement.7,8 In our case, imaging findings were compatible with those of previous Western reports.

The diagnosis of an extramedullary plasmacytoma is based on evidence of a monoclonal plasma cell tumor outside the bone marrow. In the present case, we performed EUS-FNA of the pancreas. EUS-FNA has an excellent safety profile regard-ing the risk of pancreatitis, bleeding, and perforation. Major complications have been reported in 2.5% of 355 patients who underwent EUS-FNA for a solid pancreatic mass.9 Potential complications include acute pancreatitis, infection, and seda-tion-related complications; no deaths have been reported.10 Seeding of the needle track with tumor cells is rare. Previous studies reported that the risk of seeding of pancreatic adeno-carcinoma via EUS-FNA is 2.2%, which is low compared with that (16.3%) of CT or transabdominal ultrasonography-guid-ed percutaneous biopsy.11,12 Moreover, seeding of a pancreatic extramedullary plasmacytoma during EUS-FNA is yet to be reported.

In conclusion, we safely performed EUS-FNA in a patient with pancreatic plasmacytoma, and were able to provide an accurate visualization and histopathologic diagnosis. Although there are approximately three case reports describing pancre-atic involvement of multiple myeloma diagnosed by EUS-FNA in the English language literature,7,13,14 to the best of our knowledge, this is the first time it is reported in Korea.

Fig. 5. Endoscopic ultrasonography-guided fine needle aspiration cytology (×400) shows (A) a predominantly monomorphic population of plasma cells with (B) positivity for CD138.

A B

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Conflicts of InterestThe authors have no financial conflicts of interest.

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